DESCRIPTION: This proposal seeks funds to support the 2007 FASEB Research Conference on Assembly of the Mitochondrial Respiratory Chain. The principal objective is to inaugurate a new FASEB conference on a rapidly expanding area of mitochondrial biology. The meeting will bring together scientists, ranging from established investigators to graduate students, to promote the exchange of scientific ideas among scientists interested in diverse aspects of mitochondrial biology and mitochondrial dysfunction in human disease, with a view to encouraging collaborative approaches and cross-field stimulation. Funds are requested to support trainees and junior faculty who would benefit from attendance at this conference. The timing of this conference is significant, as interest in mitochondrial biology and dysfunction has surged. Seminal advances have occurred in recent years on physiological processes within the organelle such as the dynamics of morphology, protein import into sub-compartments, identification of supercomplexes, and interorganelle communication. Insight has been gleaned from patients with cardiomyopathy, hepatopathy, and neurodegenerative disease on assembly and regulation. The biochemical defect in the majority of the patients results from a defect in assembly and this can lead to a myriad of clinical phenotypes ranging from ataxia, seizures, blindness, myoclonus, dystonia, sideroblastic anemia, deafness, and diabetes. An understanding of the structure, assembly and function of the respiratory chain is thus a highly significant topic. Sessions will deal with the structure and dynamics of the mitochondrion, the organization of respiratory chain components, transcriptional and translational regulatory circuits controlling mitochondrial biogenesis and function, mitochondrial-nuclear and mitochondrial- cytoplasm cross talk, mitochondrial protein sorting and its role in disease, assembly of individual respiratory complexes, cofactor biogenesis and cristae formation. The novelty of the Assembly of the Mitochondrial Respiratory Chain lies in the unique focus on biogenesis of the respiratory chain components and respiratory dysfunction in humans. No other meeting has attempted this. The 2007 FASEB conference Assembly of the Mitochondrial Respiratory Chain is designed to promote the exchange of scientific ideas among scientists interested in diverse aspects of mitochondrial respiration and biogenesis of the respiratory complexes. Interest in mitochondrial biology has surged in recent years, so the timing of this conference is significant in highlighting advances made in mitochondrial dynamics and morphology, inner membrane biogenesis and inter-organelle communication. Mitochondria play a key role in apoptosis, lipid metabolism, energy production, thermogenesis, iron metabolism and other catabolic and anabolic pathways. Mitochondrial dysfunction contributes to numerous inherited and acquired human diseases and the ageing process. Insight has been gleaned from patients with cardiomyopathy, hepatopathy, and neurodegenerative disease on assembly and regulation. Defects in assembly of the respiratory chain can lead to a myriad of clinical phenotypes ranging from ataxia, seizures, blindness, myoclonus, dystonia, sideroblastic anemia, deafness, and diabetes. Thus, an understanding of the structure, assembly and function of the respiratory chain is thus a highly significant topic. The novelty of the Assembly of the Mitochondrial Respiratory Chain lies in the unique focus on biogenesis of the respiratory chain components and respiratory dysfunction in humans. No other meeting has attempted this. [unreadable] [unreadable] [unreadable]